Lymphomatoid granulomatosis: A clinicopathologic study of four cases

Four cases of lymphomatoid granulomatosis were studied. One case, previously reported, has had a prolonged remission of 8 years' duration. In one case, the course was rapid and progressive, and the patient died 2 months after the onset of the disease. In two other patients, the disease appears to be arrested effectively, both clinically and radiographically, by administration of corticosteroids. In the fatal case, postmortem examination revealed a typical angiocentric and destructive polymorphous lymphoreticular infiltrate in the lungs, kidneys, and adrenal glands. The diagnosis was made on specimens obtained from three patients by open thoracotomy. An adequate specimen is mandatory for diagnosis and thoractomy is indicated. To be considered in the differential diagnosis are Wegener's granulomatosis, the limited form of Wegener's granulomatosis, lymphoma, allergic granulomatosis, lymphocytic interstitial pneumonia, plasma cell granuloma, and infectious granuloma.